Neuropathology of animal prion diseases

article

Orge, Leonor
Lima, Carla
Machado, Carla
Tavares, Paula
Mendonça, Paula
Carvalho, Paulo
Silva, João
Pinto, Maria de Lurdes
Bastos, Estela
Pereira, Jorge Cláudio
Anjo, Nuno Gonçalves
Gama, Adelina
Esteves, Alexandra
Alves, Anabela
Matos, A.C.
Seixas, Fernanda
Silva, Filipe
Pires, Isabel
Figueira, L.
Vieira-Pinto, Madalena
Sargo, Roberto
Pires, Maria dos Anjos

Orge, L.; Lima, C.; Machado, C.; Tavares, P.; Mendonça, P.; Carvalho, P.; Silva, J.; Pinto, M.; Bastos, E.; Pereira, J.; Gonçalves-Anjo, N.; Gama, A.; Esteves, A.; Alves, A.; Matos, A.; Seixas, F.; Silva, F.; Pires, I.; Figueira, L.; Vieira-Pinto, M.; Sargo, R.; Pires, M. Neuropathology of Animal Prion Diseases. Biomolecules 2021, 11(3), 466; https://doi.org/10.3390/biom11030466.

10.3390/biom11030466

Neuropathology of animal prion diseases

Transmissible Spongiform Encephalopathies
PrPSc
Chronic wasting disease
Prion diseases

info:eu-repo/grantAgreement/FCT/9471 - RIDTI/PTDC%2FCVT-CVT%2F29947%2F2017/PT

2022-01-05T12:10:46Z
2022-01-05T12:10:46Z
2021

Transmissible Spongiform Encephalopathies (TSEs) or prion diseases are a fatal group of infectious, inherited and spontaneous neurodegenerative diseases affecting human and animals. They are caused by the conversion of cellular prion protein (PrPC) into a misfolded pathological isoform (PrPSc or prion- proteinaceous infectious particle) that self-propagates by conformational conversion of PrPC. Yet by an unknown mechanism, PrPC can fold into different PrPSc conformers that may result in different prion strains that display specific disease phenotype (incubation time, clinical signs and lesion profile). Although the pathways for neurodegeneration as well as the involvement of brain inflammation in these diseases are not well understood, the spongiform changes, neuronal loss, gliosis and accumulation of PrPSc are the characteristic neuropathological lesions. Scrapie affecting small ruminants was the first identified TSE and has been considered the archetype of prion diseases, though atypical and new animal prion diseases continue to emerge highlighting the importance to investigate the lesion profile in naturally affected animals. In this report, we review the neuropathology and the neuroinflammation of animal prion diseases in natural hosts from scrapie, going through the zoonotic bovine spongiform encephalopathy (BSE), the chronic wasting disease (CWD) to the newly identified camel prion disease (CPD).
info:eu-repo/semantics/publishedVersion

openAccess
http://creativecommons.org/licenses/by/4.0/

eng